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A case of Letterer-Siwe disease in a 2 year old boy manifesting such classic signs described
by Siwe as, hepatosplenome^aly, swelling of lymph node, bleeding tendency, anemia, fever
and bony skull defects is presented.
The diagnosis was confirmed by clinical symptoms, laboratory data, histopathologic and
radiologic fingings.
The authors report a fatal case which was treated in this hospital with review of pertinent
literatures.
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